Vulvar leiomyosarcoma

　　1. Etiology

　　Mainly from the smooth muscle cells of the vulvar muscle layer or the smooth muscle fibers of the vulvar vascular wall, a few from the malignant transformation of vulvar leiomyoma.

　　2. Pathogenesis

　　The tumor is a grayish-white mass with unclear boundaries, showing infiltrative growth. Some may have a pseudocapsule, with the section showing gray-red or gray-brown, delicate like fish meat, often with focal or patchy hemorrhagic necrosis. Under the light microscope, there is proliferation of smooth muscle cells, with varying cell size and shape, disordered arrangement, large, deeply stained nuclei, prominent nucleoli, unclear nuclear membranes, and visible tumor giant cells. Some may present as epithelioid or mucinous type. The tumor cells of low-differentiated smooth muscle sarcoma have obvious atypicality, and pathological mitotic figures are common, with tumor cells mainly polymorphic tumor cells or mainly round cells. The tumor cells can infiltrate the surrounding muscle layer, blood vessels, and so on. However, high-differentiated smooth muscle sarcoma lacks obvious malignant histological characteristics, similar to atypical smooth muscle tumors, so Tavassoli et al. (1979) proposed that the diagnosis of vulvar smooth muscle sarcoma must have the following conditions: mass diameter ≥5cm, nuclear mitotic figures ≥5/10HPF, infiltrative margin. Nielsen et al. (1996) added moderate to severe nuclear atypicality to these three conditions and believed that if three or more of the four conditions are met, it is a smooth muscle sarcoma, if only two conditions are met, it is an atypical smooth muscle tumor, and if one or none are met, it is a common type of smooth muscle tumor. Nucci et al. (2000) suggested that coagulative necrosis should also be included in the diagnostic criteria.

　　Since nuclear mitotic figures play a very important role in the diagnosis of benign and malignant smooth muscle tumors, at least 10 to 15 sections should be made from different planes, and at least 4 to 5 groups of 10 consecutive high-power fields with the most active nuclear mitotic figures should be counted. The highest count group determines the number of mitotic figures of the tumor. At the same time, it is necessary to differentiate the mitotic figures from nuclear atrophy, folding, abnormal shape, or deeply stained chromatin. Electron microscopy can see longitudinal arrangement of intermediate filaments, dense spots, many pinocytotic vesicles, and partial or complete basement membrane surrounding each cell.

　　Masson staining shows red myofibrils in the cytoplasm, and PTAH staining shows purple. The smooth muscle-specific actin SMA, muscle-specific actin MSA, and vimentin are all positive, with most desmin, estrogen receptor ER, and progesterone receptor PR positive. It can also be positive for calponinhl and calcium-binding protein hcaldesmin.

　　There are reports that the recurrence of tumors is related to the following factors: tumor > 5cm; invasion at the surgical margin; and showing ≥5 mitotic figures/HP under the microscope.

　　0.4%-0.8% of uterine fibroids may undergo malignant transformation into uterine sarcoma. The malignant transformation of uterine fibroids often occurs in women aged 40-50, with rapid growth of fibroids or vaginal bleeding. The sarcoma transformation is manifested as a vulvar mass, which can be large but with clear boundaries, and the skin surface can be seen with venous dilation. Due to surface abrasion, it may lead to ulceration, and in addition to the weakened resistance and immunity of cancer patients, it may cause hemorrhage and infection. There is a possibility of concurrent sarcoma transformation.

　　1, Symptoms

　　Initially, the mass is small, located under the skin, and may have no symptoms, often manifested as a slowly growing painless mass. Some symptoms are not obvious, and patients may seek medical attention because they accidentally touched the tumor. They may also remain unchanged for several years. Patients often seek medical attention due to masses, bleeding, and pain.

　　2, Signs

　　Vulvar masses are mostly seen in the deep soft tissue around the labia majora, the labial ligament, and occasionally occur in the clitoris, mons pubis, and perineum. The size of the mass is 2-16cm, usually 5-10cm, round or elliptical, solitary or multiple solid nodular masses. In the early stage, the surface skin of the mass is intact. As the tumor progresses, the skin is involved, and symptoms such as congestion and ulceration appear. In the late stage, the tumor may invade deep tissues and become fixed at the pubic bone, ischium, or appear distant metastasis.

　　Prognosis: Vulvar smooth muscle tumor is prone to local recurrence, most patients have recurrence within 2 years after surgery, and more than 80% of the复发 patients will eventually have lung metastasis. However, there are also reports of recurrence after 10 years of surgery, so long-term follow-up is needed. The related factors of the prognosis of vulvar soft tissue sarcoma include:

　　1, Related to the surgical method; simple tumor resection has a local recurrence rate of up to 80%, while radical vulvectomy has only 20%.

　　2, Tumor size and tissue differentiation degree; tumor diameter > 5cm, the edge shows infiltrative growth rather than expansive growth, mitotic figures > 10 per 10 high-power fields (HPF), which are the most dangerous factors for poor prognosis.

　　1, Secretion examination, tumor marker examination, and detection of tumor molecular marker P53 tumor suppressor gene.

　　2, For those with infiltrated skin or skin ulcers, tissue biopsy can be taken; for those with intact skin, needle aspiration biopsy or puncture biopsy can be performed, or incisional biopsy or excisional biopsy can be done.

　　One, Dietetic Recipe for Vulvar Smooth Muscle Tumor

　　1, Ku Shen Egg: 2 eggs, 60 grams of brown sugar, 60 grams of Ku Shen. Boil Ku Shen to extract the juice, add the beaten eggs and brown sugar, and cook until done, eat the egg and drink the soup. Once a day, for 6 days as a course of treatment. It has the effects of clearing heat and detoxifying, drying dampness, and healing sores, and can be used for the prevention and treatment of vulvar ulcers.

　　2, Long Dan Cao Egg: 10 grams of Long Dan Cao, 3 eggs, 30 milliliters of honey. Boil Long Dan Cao, remove the residue, beat the eggs to make a nest egg, add honey, and take it on an empty stomach, once a day, for 5 days as a course of treatment. It has the effect of clearing heat, removing dampness, and healing sores.

　　3. Cabbage and mung bean sprout drink: One root of cabbage, 30 grams of mung bean sprouts. Clean the cabbage root and slice it, wash the mung bean sprouts and put them in a pot with water, boil for 15 minutes, remove the dregs and take the juice, drink it as tea, without regard to time. It has the effect of clearing heat and removing dampness and can be used for the treatment of vulvar ulcers.

　　4. Rock sugar winter melon soup: 30 grams of winter melon seeds, 30 grams of rock sugar. Clean the winter melon seeds, grind them into coarse powder, add rock sugar, pour one bowl of boiling water, place it in an earthen pot, and simmer over low heat. Take twice a day, for several days in a row. It has the effect of clearing heat and dampness and can be used for the treatment of vulvar itching.

　　5. Cang'er pork stomach: One pork stomach, 30 grams of Cang'erzi, 10 grams of Fructus psoraleae. Clean the pork stomach, add water, and boil with the latter two herbs together. Discard the medicine and eat the stomach. It has the effect of kidney-tonifying and wind-relieving itching. It is used for the prevention and treatment of atrophic vulvar leukoplakia.

　　6. Goji berry porridge: 20 grams of goji berries, an appropriate amount of rice. Cook the porridge for consumption.

　　Second, Foods that are good for the body in vulvar leiomyosarcoma

　　1. Eat more foods that have anti-vulvar tumor and leukoplakia effects, such as sesame, almond, wheat, barley, loofah, black chicken, squid, black mamba, pork pancreas, chrysanthemum, umeboshi, peach, lychee, shepherd's purse, chicken blood, eel, abalone, crab, horseshoe crab, sardine, clam, tortoise shell.

　　2. To relieve pain, eat horseshoe crab, red, lobsters, clams, sea cucumber, tiger fish, beetroot, mung bean, radish, chicken blood.

　　3. To relieve itching, eat amaranth, cabbage, rapeseed, taro, kelp, nori, chicken blood, snake meat, pangolin.

　　4. To enhance physical fitness and prevent metastasis, eat silver ear, black fungus, mushrooms, hedgehog mushrooms, chicken gizzards, sea cucumber, Job's tears, walnuts, crabs, lizard, needlefish, etc.

　　5. After vulvar leiomyosarcoma surgery, it may consume Qi and injure blood, so it is advisable to eat foods that tonify Qi and nourish blood, such as jujube, longan, mung bean, glutinous rice, lychee, mushrooms, carrots, quail eggs, lotus root powder, beans, etc.

　　6. Radiotherapy after vulvar leiomyosarcoma surgery: It may consume Yin and damage body fluid, so it is advisable to eat foods that nourish Yin and body fluid, such as spinach, minor greens, lotus root, radish, watermelon, banana, grapes, sea cucumber, sugarcane, lily, etc.

　　7. Chemotherapy after vulvar leiomyosarcoma surgery: It may damage both Qi and blood, so it is advisable to eat foods that nourish Qi and blood, such as black fungus, mushrooms, walnuts, mulberries, Job's tears porridge, jujube, longan, sea cucumber, etc.

　　Third, Foods to avoid for vulvar leiomyosarcoma

　　1. Avoid stimulants such as coffee.

　　2. Avoid spicy and刺激性 foods such as scallion, garlic, ginger, and cassia bark.

　　3. Avoid fatty, fried, moldy, and salted foods.

　　4. Avoid irritants such as rooster and pork head meat.

　　5. Avoid seafood.

　　6. Avoid warm foods such as mutton, dog meat, chive, pepper, etc.

　　7. Avoid smoking and drinking alcohol.

　　Precautions before the treatment of vulvar leiomyosarcoma

　　First, Prevention

　　Regular physical examinations, early detection, early treatment, and follow-up care should be done well.

　　Second, Western medical treatment methods for vulvar leiomyosarcoma

　　Vulvar sarcoma is primarily treated with surgery, and the addition of anticancer chemotherapy or radiation therapy may improve efficacy.

　　1. Surgical treatment involves radical vulvectomy and inguinal lymph node dissection. The range of resection of the primary focus must be sufficient; insufficient resection often leads to local recurrence. If the inguinal lymph nodes are positive, lymph node dissection in the iliac pelvic area is performed. Excisional surgery or conservative surgery is used, with 80% local recurrence.

　　2. For sarcomas with slightly later stages and active nuclear division in the tissue, the combination of radical surgery and chemotherapy before and after surgery can improve the prognosis.

　　(1) Currently, the commonly used anti-cancer chemotherapy regimens for the treatment of soft tissue sarcomas include:

　　①VAC regimen: Vincristine (vincristine) 1.5mg, intravenous injection, days 1, 8; Actinomycin or Actinomycin D (Actinomycin) 400-600μg, intravenous injection, days 1-4; Cyclophosphamide (cyclophosphamide) 300mg, intravenous injection, days 1, 4, 8. Repeated every 3-4 weeks, but depending on the recovery of bone marrow function. It has been reported that this regimen can extend the survival period by 4-5 times when treating pelvic sarcoma.

　　②ADIC regimen: Doxorubicin (Adriamycin) 60mg, intravenous infusion, day 1; Dacarbazine (DTIC) 250mg, intravenous infusion, days 1-5. Efficacy rate 42%.

　　③CYVADIC regimen: Cyclophosphamide 500mg, intravenous infusion, day 2; Vincristine 1.5mg, intravenous injection, days 1, 8; Dacarbazine (DTIC) 250mg, intravenous infusion, day 2. The interval between courses is 4 weeks. Efficacy rate 47%.

　　(2) For patients with localized malignant lymphoma, surgery is performed first, followed by chemotherapy, commonly using the regimen:

　　①COP regimen: Cyclophosphamide 800mg, intravenous injection, days 1 and 15; Vincristine 1.4mg, intravenous injection, day 1; Prednisone (Prednisone) 100mg, oral, days 1-5. One course repeated every 3 weeks, with an efficacy rate over 80%.

　　②CHOP regimen: Cyclophosphamide 750mg/?, intravenous infusion, day 1; Doxorubicin (Adriamycin) 50mg, intravenous infusion, day 1; Vincristine 1.4mg/?, intravenous injection, day 1; Prednisone (Prednisone) 100mg, oral, days 1-5. One course repeated every 3 weeks, with an efficacy rate over 90%.